Table 1.
Clinical characterization of spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS) patients. This table shows disease factors with a possible influence on cognitive function. Effect sizes of r = 0.10, r = 0.30, and r = 0.50 served as thresholds for small, medium and large effects [30], respectively. Abbreviations: SMA, Spinal Muscular Atrophy; ALS, Amyotrophic Lateral Sclerosis; ALSFRS-R, Amyotrophic Lateral Sclerosis Functional Rating Scale revised form; SD, standard deviation; n, number; vs., versus.
| SMA (n = 34) |
ALS (n = 34) |
SMA vs. ALS | |
|---|---|---|---|
| Age n (years) | 34 | 34 | |
| MEDIAN (RANGE) | 38.5 (19–64) | 66.0 (46–79) | |
| MEAN (SD) | 40.2 (13.1) | 65.8 (9.4) | |
| T = −9.253, df = 59.734; p < 0.001; r = 0.77 | |||
| Gender n | 34 | 34 | |
| female | 11 (32.4%) | 13 (38.2%) | |
| U = 544.00; Z = −0.504; p = 0.614; r = 0.06 | |||
| SMA type n | 34 | ||
| type 1 | 0 | ||
| type 2 | 14 (41.2%) | ||
| type 3 | 19 (55.9%) | ||
| type 4 | 1 (2.9%) | ||
| Disease onset n (at age in years) | 34 | 32 | |
| MEDIAN (RANGE) | 1.8 (0–47) | 63.5 (41–77) | |
| MEAN (SD) | 6.5 (9.3) | 63.4 (9.4) | |
| U = 2.00; Z = −6.957; p < 0.001; r = 0.85 | |||
| Disease duration n (years) | 34 | 33 | |
| MEDIAN (RANGE) | 32.5 (3–62) | 1.4 (0–18) | |
| MEAN (SD) | 33.8 (13.2) | 2.2 (3.2) | |
| U = 9.50; Z = −6.920; p < 0.001; r = 0.85 | |||
| Years of education n | 34 | 34 | |
| MEDIAN (RANGE) | 16.0 (10–24) | 12.5 (8–21) | |
| MEAN (SD) | 16.3 (3.5) | 13.1 (2.8) | |
| U = 272.00; Z = −3.762; p < 0.001; r = 0.46 | |||
| Education level n | 34 | 19 | |
| Intensified secondary education (German Abitur) | 21 (61.8%) | 6 (31.6%) | |
| Extensive secondary education (German Realschule) | 10 (29.4%) | 4 (21.1%) | |
| Basic secondary education (German Hauptschule) | 2 (5.9%) | 9 (47.4%) | |
| Other | 1 (2.9%) | 0 (0%) | |
| ALSFRS-R n | 34 | 29 | |
| MEDIAN (RANGE) | 30.0 (10–48) | 33.0 (16–41) | |
| MEAN (SD) | 30.8 (10.8) | 31.3 (7.0) | |
| U = 493.00; Z = 0.000; p > 0.999; r = 0.00 | |||
| Ambulatory status n | 34 | 27 | |
| ambulatory | 9 (26.5%) | 16 (59.3%) | |
| manual/electric wheelchair use (part-time, always) | 25 (73.5%) | 11 (40.7%) | |
| Care level 1 n | 32 | 23 | |
| 0 | 11 (34.4%) | 6 (26.1%) | |
| 1 | 1 (3.1%) | 6 (26.1%) | |
| 2 | 0 (0%) | 5 (21.7%) | |
| 3 | 5 (15.6%) | 4 (17.4%) | |
| 4 | 9 (28.1%) | 1 (4.3%) | |
| 5 | 6 (18.8%) | 1 (4.3%) |
1 Care level: classification at the care level according to the German health care system, with higher levels indicating a greater loss of autonomy and a need for more individual support (care level 1 = minor impairment of individual autonomy, care level 5 = severe impairment of individual autonomy with special care requirements); also see [31,32].